Hitchins R, Naughton L, Kerlin P, Cobcroft R
Department of Clinical Haematology, Princess Alexandra Hospital, Brisbane.
Pathology. 1988 Jan;20(1):59-61. doi: 10.3109/00313028809085198.
A 65-year-old male developed a rapidly progressive disease characterized by severe hemolysis, with spur cells (acanthocytes) and liver disease. Autopsy findings were consistent with a diagnosis of idiopathic hemochromatosis. Investigation of the patient's family uncovered four out of five first degree relatives with significantly raised serum ferritin levels. A sister had biopsy proven hemochromatosis. Spur cell anemia is a recognized, though rare, complication of alcoholic liver disease and indeed the patient had a regular alcohol intake of up to 50 g daily. Although the alcohol intake could have contributed to the formation of spur cells, the possible association with hemochromatosis should be considered. A diagnosis of hemochromatosis has important implications for family members.
一名65岁男性患有一种快速进展的疾病，其特征为严重溶血、出现棘状细胞（锯齿状红细胞）以及肝脏疾病。尸检结果符合特发性血色素沉着症的诊断。对该患者家族的调查发现，其五名一级亲属中有四人血清铁蛋白水平显著升高。一名姐妹经活检证实患有血色素沉着症。棘状细胞贫血是酒精性肝病一种公认但罕见的并发症，实际上该患者每日有规律地摄入高达50克酒精。尽管酒精摄入可能促使棘状细胞形成，但应考虑其与血色素沉着症的可能关联。血色素沉着症的诊断对家庭成员具有重要意义。
